麻豆传媒

Former NFL wide receiver Randy Moss announced last week that he underwent a 6-hour surgery for a rare and aggressive gastrointestinal cancer.

Moss, 47, detailed in an that a tumor was found in his bile duct, "right between the pancreas and the liver."

He said he was hospitalized for 6 days and had undergone a Whipple procedure, which is most commonly used for early-stage cancers located near the pancreas. During , also known as pancreaticoduodenectomy, surgeons remove parts of the bile duct, stomach, small intestine, and the head of the pancreas. Part of the small intestine is then sewn to the remainder of the bile duct, stomach, and pancreas so pancreatic enzymes and bile can continue to flow into the small intestine.

"From an oncological standpoint, the goal is not just to pluck out the cancer, but to have a clear zone of clean margins around the area of involvement, as well as the ability to resect/remove the draining lymph nodes," Pashtoon Kasi, MD, MS, medical director of gastrointestinal medical oncology at City of Hope Orange County in Irvine, California, told 麻豆传媒 via email.

"There may or may not be involvement of nearby structures," he added. "But the surgery is done to ensure a wide, clean margin. It is done with curative intent." As the name might suggest, pancreaticoduodenectomy is also used for cancers of the pancreas or duodenum.

Moss , half of those years with the Minnesota Vikings, the team that drafted him and where he won offensive rookie of the year honors in 1998. He went on to play for the Oakland Raiders, New England Patriots, Tennessee Titans, and San Francisco 49ers.

He appeared in two Super Bowls -- with the Patriots and 49ers, both losses -- and finished his career with 156 receiving touchdowns () and 15,292 receiving yards (fourth all time). He was inducted into the Pro Football Hall of Fame in 2018.

A Rare and Aggressive Cancer

Bile duct or biliary tract cancers "arise in the plumbing of the liver draining the bile," said Kasi. "These aggressive tumors of the bile ducts are also referred to as cholangiocarcinoma -- extrahepatic when it is involving the ducts/tubes outside of the liver, and intrahepatic when it is in the drainage system/smaller ducts inside the liver."

According to the American Cancer Society, more than 40,000 patients are diagnosed with liver or biliary tract cancers each year, with cholangiocarcinomas .

Incidence has been on the rise, partly due to risk factors, "but also because we are able to diagnose these patients better, and accurately say that these are cholangiocarcinomas," said Kasi.

"Previously, and even now, some patients with cholangiocarcinoma start their journey as 'cancer of unknown primary' -- or CUP," he said. Incidence of CUP has been declining, but coincides with a rise in cholangiocarcinoma.

Patients with biliary tract cancers may present with symptoms such as jaundice, dark urine, clay-colored stool, abdominal pain, fever, pruritus, nausea and vomiting, and weight loss. Diagnostic evaluation includes liver function tests and other laboratory testing, along with various imaging methods -- CT, MRI, ultrasound.

Moss said his urine had started to become discolored and that he had to have a stent put in his liver on Thanksgiving so doctors could determine where his cancer was.

Viewers of "Sunday NFL Countdown," a pregame show where Moss is one of the cast members, had also noticed a yellowing of Moss' eyes in recent weeks.

"I didn't think I would ever be in a position like this, as healthy as I thought I was," said Moss, who encouraged people to get checked if they have unusual symptoms, "especially Black men. You got to be able to check your ego at the door."

Treatment

A majority of patients with cholangiocarcinomas have unresectable disease at diagnosis, which is associated with worse prognosis. But Moss' cancer appears to have been diagnosed at an early stage, given that surgical resection was an option. He said he would undergo chemotherapy and radiation therapy as well.

"Typically, adjuvant chemotherapy is recommended for all patients with cholangiocarcinoma if medically there are no contraindications to it," said Kasi. "Radiation is done in select high-risk patients where the margins may be close or if the tumor is extensive, and/or involvement of lymph nodes. While we cannot guess the staging, we do know that we do all of this with curative intent in mind. And there might be risk features necessitating the use of radiation alongside standard chemotherapy."

Randomized trials testing adjuvant chemotherapy have not been definitive, but have shifted practice in favor of postoperative treatment to address residual or microscopic disease. The best evidence in support of adjuvant chemotherapy may be from the BILCAP trial, where median overall survival numerically improved from 36.4 months with surgery alone to 51.1 months with the addition of adjuvant capecitabine in the intent-to-treat population, though fell short of statistical significance. A per-protocol analysis did support a survival improvement, however.

Advances in Care

"The biggest advancements have been to realize that cholangiocarcinoma is not just one disease," Kasi said. "Genetic and molecular testing is giving us insights into the fact that this is a heterogeneous group of diseases with different treatment options and biology/prognosis."

In the locally advanced or metastatic setting, immunotherapy has recently been incorporated into the first-line setting following the approvals of and , in combination with chemotherapy. The two checkpoint inhibitors demonstrated improved overall survival in phase III studies.

Several targeted agents are also approved for previously treated patients with advanced or metastatic disease -- pemigatinib (Pemazyre) and futibatinib (Lytgobi) for tumors associated with FGFR2 gene fusions or other rearrangements, for tumors harboring IDH1 mutations, and zanidatamab (Ziihera) for HER2-positive cancers, along with for BRAF V600E-mutant cholangiocarcinomas.

Some bile duct cancers are hereditary as well, noted Kasi.

"In particular, we screen with panel-based testing all our patients with cholangiocarcinomas, and look for the breast cancer family of genes (BRCA1/2 genes) that are not uncommon in patients with cholangiocarcinoma," he said. "Key is to make sure we do comprehensive molecular somatic and germline genetic testing for all our patients with biliary tract cancers/cholangiocarcinoma as well as pancreas cancers."

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